江汉大学学报(自然科学版) ›› 2018, Vol. 46 ›› Issue (2): 167-175.doi: 10.16389/j.cnki.cn42-1737/n.2018.02.010

• 医学 • 上一篇    下一篇

心肌淀粉样变性临床诊治新进展

沈燕玲1,苏文*2,田立群2   

  1. 1.江汉大学 医学院,湖北 武汉 430056;2.武汉市第一医院,湖北 武汉 430022
  • 出版日期:2018-04-28 发布日期:2018-04-12
  • 通讯作者: 苏文
  • 作者简介:沈燕玲(1992—),女,硕士生,研究方向:内科。

Research Progress of Diagnosis and Treatment of Cardiac Amyloidosis

SHEN Yanling1,SU Wen*2,TIAN Liqun2   

  1. 1.School of Medicine, Jianghan University, Wuhan 430056, Hubei, China; 2. Wuhan No.1 Hospital, Wuhan 430022, Hubei, China
  • Online:2018-04-28 Published:2018-04-12
  • Contact: SU Wen

摘要: 心肌淀粉样变性是因为错误折叠蛋白质片段沉积于心肌细胞外基质所致的一类疾病,淀粉样蛋白质可来源于单克隆免疫球蛋白轻链、运载甲状腺素蛋白(TTR)等,患者可表现为心瓣膜病、心肌病、心衰及各种心律失常等症状。目前已有多种无创诊断手段,但心肌活检仍然是诊断心脏淀粉样变性的唯一金标准。过去的治疗重点在于改善患者心衰、心律失常症状,如今,先进的化疗方案、免疫治疗手段、干细胞移植以及调控基因表达等已兴起。综述了近年来心肌淀粉样变诊断、治疗研究的新进展。

关键词: 心肌淀粉样变性, 分型, 诊断, 治疗

Abstract: Cardiac amyloidosis is a kind of disease which results from deposition misfolded protein fragments on myocardial extracellular matrix. These protein may derive from monoclonal immunoglobulin, transthyretin (TTR), etc. The manifestations of patients with cardiac amyloidosis may perform as valvulopathy, cardiomyopathy, heart failure and various kinds of arrhythmia etc. In spite of multitudinous noninvasive diagnostic methods, myocardial biopsy remains the gold standard in the diagnosis of cardiac amyloidosis. Actually, the previous treatments focus on improving the symptoms of heart failure and arrhythmia, at present, advanced chemotherapy regimens, immune therapy, stem cell transplantation, regulation of gene expression and more progressive methods are rising up. This paper describes the new research progress of diagnosis, treatment of myocardial amyloidosis.

Key words: cardiac amyloidosis, classification, diagnosis, treatment

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